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. 2021 Oct;46(5):485-496.
doi: 10.1007/s00059-021-05054-x. Epub 2021 Sep 6.

[Cardiac amyloidosis and aortic valve stenosis]

[Article in German]
Sophiko Erbel-Khurtsidze  1 , Rainer Seibel  2 , Detlef Moka  3 , Ka T J A Brandt-Mainz  3 , Raimund Erbel  4 , Artur Lichtenberg  5
Affiliations

[Cardiac amyloidosis and aortic valve stenosis]

[Article in German]
Sophiko Erbel-Khurtsidze et al. Herz. 2021 Oct.

Abstract
in English, German

Aortic valve stenosis in old age has become a topic of interest for cardiology and cardiac surgery after the development of transvascular and transluminal minimally invasive techniques for aortic valve implantation. The observation of amyloid deposits in surgically excised valvular material led to the diagnostics of amyloidosis of the myocardium, which was discovered in up to 20% of the patients who underwent valve implantation. Clinical signs of cardiac amyloidosis, such as carpal tunnel syndrome and ruptured distal biceps tendon should be taken into account. In addition to the electrocardiogram (ECG), echocardiogram and magnetic resonance imaging, 99mtechnetium bone scintigraphy plays a key diagnostic role. The simultaneous occurrence of severe aortic valve stenosis and amyloidosis explains the special hemodynamic situation of a low gradient with low blood flow in high-grade valve stenosis. The interventional or surgical valve implantation improves the prognosis for these patients, similarly to aortic valve stenosis alone, followed by a specific pharmaceutical treatment depending on the type of amyloidosis.

Die Aortenklappenstenose im höheren Alter ist in den Blickpunkt der Kardiologie und Herzchirurgie gerückt, nachdem transvaskuläre und transluminale minimal-invasive Verfahren zur Klappenimplantation entwickelt worden waren. Die Beobachtung von Amyloidablagerungen in operativ entfernten Klappen führte zur Diagnostik der Amyloidose des Myokards, die bei bis zu 20 % der Patienten, die zur Klappenimplantation kommen, aufgedeckt wurde. Klinische Zeichen wie Karpaltunnelsyndrom oder Bizepssehnenruptur sind zu beachten. Eine wichtige diagnostische Rolle spielt neben EKG, Echokardiogramm und Magnetresonanztomographie die 99mTechnetium-Knochenszintigraphie. Das Zusammentreffen von Aortenklappenstenose und Amyloidose erklärt die besondere hämodynamische Situation eines niedrigen Gradienten, mit niedrigem Blutfluss bei hochgradiger Klappeneinengung. Die interventionelle oder chirurgische Klappenimplantation verbessert die Prognose dieser Patienten ähnlich wie bei reiner Aortenklappenstenose, gefolgt von einer spezifischen medikamentösen Therapie in Abhängigkeit vom Amyloidosetyp.

Keywords: Amyloid; Aortic valve; Carpal tunnel syndrome; Magnetic resonance imaging; Scintigraphy.

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References

Literatur

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